Juvenile pseudorheumatism

Description:

Juvenile pseudorheumatism - the chronic inflammatory disease of joints at children up to 16 years with an unknown etiology and a difficult pathogeny which is characterized by steadily progressing current and followed at some internals sore with involvement, quite often coming to an end with an invalidism.

Among rheumatic diseases of children's age the juvenile pseudorheumatism wins first place on prevalence. The disease is observed in various regions of the globe with a frequency from 0,05 to 0,6% in population. Primary incidence also fluctuates in considerable limits, making from 6 to 19 cases on 100 000 children's population.

Symptoms of the Juvenile pseudorheumatism:

the linichesky picture of a juvenile pseudorheumatism is various. The beginning of a disease can be acute or subacute. At the acute beginning body temperature usually increases, there is a morbidity, and then swelled in one or several joints, is more often than symmetric. However symmetry of defeats sometimes becomes obvious not at once, and within several days or weeks from an onset of the illness. Large joints - knee, talocrural, radiocarpal are surprised, as a rule, but sometimes from the very beginning of a disease small joints of hands and legs suffer (metatarsophalangeal, interphalanx). Typically for a juvenile pseudorheumatism damage of joints of cervical department of a backbone. All joints are sharply painful, edematous, in rare instances skin around them is hyperemic. Body temperature gradually increases and can reach 38-39 °C. At the same time it is frequent on skin of a trunk and extremities there is polymorphic allergic rash, peripheral lymph nodes, a liver and a spleen increase. In the general blood test reveal anemia, often neutrophylic leukocytosis with a deviation to the left, increase in SOE up to 40-60 mm/h, increase in concentration of Ig, IgG is preferential.

The acute onset of the illness is usually peculiar to severe forms - a generalized joint or joint and visceral (system) form of a disease with often recurrent current and the adverse forecast. This form is observed more often at children of preschool and younger school age, but it can arise also at teenagers.

The subacute onset of the illness is characterized by less bright symptomatology. Arthritis, as a rule, begins with one joint - knee or talocrural. The joint swells, its function, sometimes even without the expressed morbidity is broken. At the child gait changes, and children up to 2 years cease to go. Observe the so-called morning constraint in joints which is expressed that the patient after a night dream feels some time difficulty at the movements in joints and self-service. He hardly gets up, his gait is slowed down. Morning constraint can proceed of several minutes to 1 h and more. Process can be limited to one joint (rhematoid monoarthritis) for a long time. Such form of a disease, especially at girls of preschool age, quite often is followed by rhematoid damage of eyes - a rhematoid uveitis, unilateral or bilateral. At rhematoid twine all covers of an eye are mentioned owing to what visual acuity sharply falls up to its total loss, and sometimes within half a year. In rare instances development of a rhematoid uveitis can precede joint process that extremely complicates timely diagnosis.

The subacute onset of the illness can proceed and with involvement in process of several joints - 2-4 is more often. Such form of a disease call oligoartikulyarny. Joint pains can be moderate, as well as exudative changes. Can be involved in process, for example, two talocrural and one knee joint and vice versa. Body temperature does not increase, a polyadenitis moderate. This form of a juvenile pseudorheumatism proceeds is more good-quality, with less frequent aggravations.

In the subsequent, when progressing a disease, two main forms - preferential joint and joint and visceral in the ratio 65-70% and 35-30% respectively are possible.

Joint and visceral form

Joint and visceral (a system form) includes five signs: persistent high fever, polymorphic allergic rash, lymphadenopathy, hepatolienal syndrome, arthralgia/arthritis. This form of a juvenile pseudorheumatism has two main options - Steel's syndrome more often developing at children of preschool age and Viselera-Fankoni's syndrome, usually observable at school students.
The recurrent current of a juvenile pseudorheumatism is long can be complicated by a secondary amyloidosis that is promoted by continuous circulation in a circulatory bed of cell-bound immune complexes. Amyloid is laid in walls of vessels, in kidneys, a liver, a myocardium, intestines that leads to disturbance of their functions. Most often the amyloidosis affects kidneys what the resistant proteinuria with development in the subsequent a chronic renal failure testifies to.

Joint form

At a joint form progressing of a juvenile pseudorheumatism leads to permanent deformation of joints with partial or full restriction of mobility in them. To 25% of children become disabled people.

Diagnosis of a juvenile pseudorheumatism often presents difficulties, especially at early stages of a disease.

Reasons of the Juvenile pseudorheumatism:

The etiology of a juvenile pseudorheumatism is unknown so far. Among its reasons consider set of various environmental factors (viral and bacterial infections, a joint injury, overcooling of an organism, insolation, administration of proteinaceous drugs, etc.). Inadequate response at patients with a juvenile pseudorheumatism "supersensitivity to various environmental factors" therefore the difficult immune response leading to development of the progressing disease forms is the cornerstone of them. A part is played also by heredo-familial predisposition to rheumatic diseases. Researches of the last decades revealed communication of a juvenile pseudorheumatism with existence at patients of a DR locus of HLA with dominance of DR4 at patients with system forms of a disease and DR5 - with preferential joint option of a disease.

Treatment of the Juvenile pseudorheumatism:

Treatment at a juvenile pseudorheumatism needs to be carried out in a complex and step by step. During the active period of a disease patients need hospitalization, in inactive - outpatient observation and sanatorium treatment. A considerable part of time patients are treated in out-patient conditions in connection with disease duration. In policlinic children continue to receive the combined therapy including drug treatment, LFK, courses of massage and physical therapy. Only prolonged and continuous treatment under control of the doctor and nurse can give positive effect.